Paeds Flashcards

1
Q

what are 3 characteristic signs of pneumonia on examination?

A

bronchial breath sounds
focal coarse crackles
dullness to percussion

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2
Q

what is the most common cause of pneumonia in children?

A

streptococcus pneumonia

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3
Q

what is the most common viral cause of pneumonia?

A

respiratory syncytial virus (RSV)

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4
Q

what is the typical x-ray finding in s. aureus pneumonia?

A

pneumatocoeles (round air filled cavities) and multilobe consolidation

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5
Q

what are 2 bacteria that are more likely to cause pneumonia in pre/un-vacinated children?

A

GBS
Haemophilus influenza

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6
Q

which bacterial pneumonia can cause extrapulmonary manifestations and what are they?

A

Mycoplasma pneumonia
Can cause erythema multiforme

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7
Q

what is the management of pneumonia?

A

1st - amoxicillin

+/- macrolide (erythro, clarithro, azithro) for atypicals or allergy

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8
Q

what is the name of the condition where people cannot convert IgM to IgG so cannot form long term immunity?

A

immunoglobulin class-switch recombination deficiency

VACCINES DONT WORK!

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9
Q

what age group is typically affected by croup?

A

6 months - 2years

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10
Q

what is the pathophysiology of croup?

A

upper resp tract Ix causing oedema of the larynx

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11
Q

what is the most common pathogenic cause of croup?

A

parainfluenza virus

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12
Q

what are 2 key features of croup caused by parainfluenza virus?

A

improves in <48 hours
responds well to dexamethasone

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13
Q

what are 3 other common pathogenic causes of croup

A

influenza
adenovirus
RSV

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14
Q

what is a possible cause of croup especially in pre/unvaxinated children?

A

diphtheria - leads to epiglottitis!!! high mortality!

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15
Q

what are 5 symptoms of croup?

A

increased work of breathing
barking cough in clusters
hoarse voice
stridor
low grade fever

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16
Q

what is the treatment for croup?

A

supportive

oral dexamethasone - single dose 150 mcg/Kg (can repeat at 12 hours)

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17
Q

what is the stepwise management of severe croup?

A

oral dexamethasone
O2
Neb budesonide
Neb adrenaline
Intubation and ventilation

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18
Q

what age is virally induced wheeze typical in?

A

<3 years

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19
Q

what is the pathophysiology of viral induced wheeze?

A

viral infection causes inflammation and oedema which reduces space for air flow in a greater proportion due to small size of child’s airways- Poiseuilles law

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20
Q

what is the management of viral induced wheeze?

A

same as acute asthma

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21
Q

what kind of wheeze is heard in asthma and viral wheeze?

A

expiratory wheeze throughout

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22
Q

what is moderate asthma?

A

peak flow >50% predicted
normal speech
otherwise well

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23
Q

what is severe asthma? (6)

A

peak flow <50% predicted
sats <92%
unable to complete sentences
signs of respiratory distress
RR >40 1-5years, >30 5+ years
HR >140 1-5 years, >125 5+ years

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24
Q

what is life threatening asthma?

A

peak flow <33% predicted
sats <92%
exhaustion and poor resp effort
hypotension
silent chest
cyanosis
altered consciousness/confusion

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25
Q

what is a physical finding on the chest wall in severe chronic asthma?

A

harrison sulus - indentation in chest wall along 6th rib can be bilateral or unilateral

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26
Q

what is the acute management of asthma/viral wheeze?

A

Escalating Bronchodilators - Salbutamol, ipratropium bromide, magnesium sulphate, aminophylline

PLUS - Steroids - prednisone (oral) or Hydrocortisone IV

ABx - if suspected

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27
Q

what is the stepwise progression of bronchodilators in acute asthma?

A

IHR/Neb salbutamol (10 puffs every 2 hours or repeat nebs every 20-30 mins)
IHR/Neb ipratropium bromide
IV magnesium sulphate
IV aminophylline

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28
Q

what on spirometry suggests an obstructive picture?

A

reversibility
FEV1 <80%, FEV1:FVC <0.7

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29
Q

what are 4 investigations for asthma?

A

spirometry
direct bronchial challenge
fractional exhaled nitric oxide
peak flow variability

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30
Q

what is the long term management of asthma over 5?

A

Reliever - SABA (salbutamol)
Preventer
1 - ICS
2 - ICS + LTRA (leukotriene receptor antagonist - montelukast)
3 - ICS + LABA (-LTRA)
4 - MART (maintenance and reliever therapy, steroid + (S)LABA)

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31
Q

which LABA can be used as a short acting agonist also?

A

folmeterol

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32
Q

can ICS cause restricted growth?

A

can reduced adult hight with long term use by up to 1cm . dose dependent effect

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33
Q

what is the most common cause of bronchiolitis?

A

Respiratory syncytial virus (RSV)

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34
Q

in what age group does bronchiolitis occur?

A

<1 year, most common <6 months can be up to 2 years

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35
Q

what are 8 typical signs of bronchiolitis?

A

coryzal symptoms - URTI
signs of resp distress
dyspnoea
tachypnoea
poor feeding
mild fever
apnoea
wheeze and crackles

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36
Q

what are 8 signs of respiratory distress?

A

raised RR
use of accessory muscles
intercostal and subcostal recession
nasal flaring
head bobbing
tracheal tugging
cyanosis
abnormal airway noise

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37
Q

what is the typical course of RSV bronchiolitis?

A

coryzal symptoms
chest symptoms 1-2 days later
worst day 3-4
symptoms usually last 7-10 days

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38
Q

what are reasons for admission in bronchiolitis?

A

<3 months, pre-existing conditions
<50-70% of normal milk intake
clinical dehydration
RR >70
O2 <92%
resp distress
apnoea
parent not able to manage/access medical help at home

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39
Q

what is the management of bronchiolitis?

A

ensure adequate intake - NG, IVs
saline nasal drops, nasal suctioning
O2
ventilation support

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40
Q

2 signs of poor ventilation of a cap gas?

A

rising pCO2
falling pH

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41
Q

what can be given as prevention of RSV infection?

A

Palivizumab

monthly injection as prevention of bronchiolitis caused by RSV given to high risk babies. Provides passive protection

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42
Q

what age does laryngomalacia occur at?

A

infants peaking at 6 months - inspiratory stridor exacerbated by lying, feeding, upset => no coinciding resp distress

problem resolves as larynx matures - around 1.5 years

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43
Q

what chromosome is CF carried on?

A

chromosome 7

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44
Q

mutations on what gene causes CF?

A

Cystic fibrosis transmembrane conductance regulatory gene

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45
Q

what channels are affected in CF?

A

chloride channels

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46
Q

what is often the first sign of CF?

A

meconium ileus - not passing meconium in 24 hours, abdo distention, vomiting

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47
Q

what are 7 causes of clubbing in children?

A

hereditary
cyanotic heart disease
infective endocarditis
CF
TB
IBD
Liver cirhosis

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48
Q

what is the diagnostic chloride concentration on sweat test for CF?

A

> 60 mmol/L

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49
Q

which are 2 bacteria which are especially difficult to treat in CF?

A

pseudomonas aeruginosa
Burkholderia cepacia

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50
Q

what Abx can be used to treat pseudomonas?

A

tobramycin nebs or oral ciprofloxacin

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51
Q

what is the management of CF?

A

multidisciplinary

chest physio
exercise
high calorie diet
CREON tablets - for pancreatic insufficiency
prophylactic flucloxacillin
Tx chest infections
bronchodilators
DNase nebs - help clear secretions
hypertonic saline nebs
vaccinations

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52
Q

what is the average life expectancy for CF?

A

47 years

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53
Q

what infection typically causes epiglottitis?

A

HiB

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54
Q

what are 8 symptoms of epiglottitis?

A

sore throat/pain swallowing
stridor
drooling
tripod position
high fever
muffled voice
scared and quiet
septic and unwell

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55
Q

what investigation can be done in suspected epiglottitis?

A

lateral xray of neck

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56
Q

what is the management of epiglottitis?

A

alert senior paediatrician and anaesthetist

ABx - cefriaxone
steroids - dexamethasone

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57
Q

what is a complication of epiglottitis?

A

epiglottic abscess

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58
Q

what is chronic lung disease of prematurity (bronchopulmonary dysplasia)?

A

typically <28 weeks gestation suffer from resp distress syndrome and require O2 or intubation and ventilation at birth. Diagnosed on CXR when infant required O2 >36 weeks gestational age

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59
Q

what are 5 features of chronic lung disease of prematurity?

A

low O2 sats
increased work of breathing
poor feeding and weight gain
crackles and wheeze
increased susceptibility to infection

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60
Q

what can reduce risk of chronic lung disease of prematurity?

A

corticosteroids (betamethasone) in premature labour mothers <36 weeks
CPAP rather than intubation
Caffeine to stimulate resp effort
avoid over-oxygenating

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61
Q

what is the most common pathogen of otitis media?

A

strep pneumoniae

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62
Q

what is the typical presentation of otitis media?

A

ear pain
reduced hearing
symptoms of URTI

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63
Q

what is the first line Abx for otitis media?

A

1 - amoxicillin 5 days

erythro, clarithro

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64
Q

what is glue ear?

A

otitis media with effusions - middle ear become full of fluid due to a blockage in the eustacion tube causing loss of hearing

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65
Q

what can be seen on otoscopy in glue ear?

A

dull tympanic membrane with air bubbles or a visible fluid level

can look normal

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66
Q

what is the natural course of glue ear?

A

usually resolves within 3 months without treatment

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67
Q

how long does it usually take grommets to fall out?

A

1 year

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68
Q

what are 3 common congenital causes of deafness?

A

maternal rubella or CMV
genetic deafness
associated syndromes - downs

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69
Q

what are 2 common perinatal causes of deafness?

A

prematurity
hypoxia during or after birth

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70
Q

what are 4 common post natal causes of deafness?

A

jaundice
meningitis and encephalitis
otitis media or glue ear
chemo

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71
Q

what is the range of normal hearing on an audiogram?

A

all readings between 0-20 dB

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72
Q

what are 3 symptoms additional to tonsillitis symptoms that can indicate quinsy?

A

trismus - unable to open mouth
change in voice (hot potato voice)
swelling and erythema surrounding tonsils

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73
Q

what is the most common pathogen in quinsy?

A

strep pyogenes (A) and Haemophilus influenzae

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74
Q

what is the management of quinsy?

A

drainage and Abx (co-amox)
?dex

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75
Q

what is preorbital cellulitis?

A

infection of upper and lower eyelid which causes red how skin swelling around eyelid and eye - distinguish from orbital cellulitis with CT scan

usually no pain or reduction in eye movements, no change in vision or abnormal pupillary response

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76
Q

what is the management of preorbital cellulitis?

A

systemic Abx

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77
Q

what is squint also known as?

A

strabismus - misalignment of the eyes causing double vision

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78
Q

what are 5 causes of squint?

A

idiopathic
hydrocephalus
cerebral palsy
space occupying lesion
trauma

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79
Q

what are 2 tests for squint?

A

cover test
herschberg’s test

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80
Q

what is the management of squint?

A

occlusive patch or atropine drops in good eye

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81
Q

what are the 3 foetal circulation shunts?

A

ductus venosus
foramen ovalae
ductus arteriosus

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82
Q

what is the ductus venosus?

A

shunt connecting umbilical vein to inferior vena cava allowing foetal blood to bypass liver

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83
Q

what is the foramen ovale?

A

shunt connecting R to L atrium which allows blood to bypass R ventricle and pulmonary circulation

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84
Q

what is the ductus arteriosus?

A

shunt connecting pulmonary artery and aorta which allows blood to bypass the pulmonary circulation

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85
Q

are atrial septal defects cyanotic?

A

no - unless R sided pressure becomes so great due to pulmonary hypertension the shunt reverses - EISENMENGER SYNDROME

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86
Q

what is eisenmenger syndrome?

A

when a shunt reverses due to pulmonary HTN causing R sided pressure > L sided pressure leading to pulmonary circulation bypass and cyanosis. develops after 1-2 years with large shunts and in adulthood with small

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87
Q

what are 4 complications of atrial septal defects?

A

stroke - with DVT - clot bypasses lungs
AF or atrial flutter
Pulmonary hypertension and r sided HF
Eismenger syndrome

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88
Q

what are 3 types of atrial septal defect?

A

Ostium secondum
patent foramen ovale
ostium primum - tend to causes Av valve defects also

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89
Q

what is the murmur in ASD?

A

mid systolic crescendo-decrescendo murmur loudest at upper left sternal border with a fixed split second heart sound

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90
Q

what are 5 typical ASD symptoms?

A

SOB
difficulty feeding
poor eight gain
LRTIs
complications - heart failure, stroke

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91
Q

what can be seen on examination in eisenmengers syndrome?

A

cyanosis
clubbing
dyspnoea
plethoric complection (due to polycythaemia)

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92
Q

what is the management of eisenmengers syndrome?

A

Heart lung transplant

Management of pulmonary HTN, polycythaemia and thrombosis

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93
Q

what genetic conditions are associated with VSDs?

A

Downs and turners

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94
Q

what 3 defects can cause eisenmenger’s syndrome?

A

ASD
VSD
patent duct arteriosus

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95
Q

what is the murmur is VSD?

A

pan-systolic more prominent at left lower sternal border in 3/4 intercostal space. May be systolic thrill

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96
Q

what are the 3 causes of pan-systolic murmur?

A

VSD
mitral regurg
tricuspid regurg

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97
Q

how long does it usually take for the ductus arteriosus to close?

A

1-3 days to stop functioning, 2-3 weeks to fully close

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98
Q

what kind of shunt is patent duct arteriosus?

A

left to right - from aorta to pulmonary vessels

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99
Q

what murmur is heard in patent duct arteriosus?

A

continuous crescendo -decrescendo machinery murmur

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100
Q

what causes an ejection systolic murmur? (3)

A

aortic stenosis
pulmonary stenosis
hypertrophic obstructive cardiomyopathy

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101
Q

what 2 conditions that cause cyanotic heart disease?

A

transposition of the great arteries
Tetralogy of fallot

Eisenmengers transformation

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102
Q

what condition is coarctation of the aorta associated with?

A

turners syndrome

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103
Q

what is the presentation of coarctation of the aorta?

A

weak femoral pulses - upper limb BP higher than lower limb
Systolic murmur below left clavicle
tachypnoea
poor feeding
grey, floppy baby

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104
Q

what is the management of coarctation of the aorta?

A

surgery
in high risk - prostaglandin E to keep ductus arteriosus open till surgery

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105
Q

what cardiac condition causes cyanosis at birth?

A

transposition of the great arteries

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106
Q

what is the management of transposition of the great arteries?

A

prostaglandins - maintain ductus arteriosus
Balloon septostomy - insert balloon catheter into foramen ovale to create largeASD
open heart surgery - arterial switch

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107
Q

what are the 4 pathologies in tetralogy of fallot?

A

Pulmonary valve stenosis
Right ventricular hypertrophy
Overriding Aorta
VSD

PROVe

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108
Q

what kind of shunt is there in tetralogy of fallot?

A

left to right - cyanotic

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109
Q

what are 4 risk factors for tetralogy of fallot?

A

rubella infection
increased age of mother
alcohol in pregnancy
diabetic mother

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110
Q

what investigations can be used for septal defects?

A

echo + doppler flow studies

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111
Q

what can be seen on chest x-ray in tetralogy of fallot?

A

boot shaped heart due to right ventricular hypertrophy

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112
Q

what are 6 manifestations of tetralogy of fallot?

A

cyanosis
clubbing
poor feeding
poor weight gain
ejection systolic murmur loudest in pulmonary area
tet spells

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113
Q

what are tet spells?

A

seen in tetralogy of fallot where shunt becomes temporarily worse causing cyanotic episode - usually during waking, exertion, crying

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114
Q

what is the management of tet spells?

A

squatting or pulling knees to chest - increases systemic vascular resistance

O2
beta blockers - relax R ventricle
IV fluids
morphine - decrease resp drive
sodium bicarb - buffer metabolic acidosis
phenylephrine infusion - increase systemic vascular resistance

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115
Q

what is the management of tetralogy of fallot?

A

Prostaglandins - maintain ductus arteriosus

surgery

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116
Q

what is ebstein’s anomaly?

A

R tricuspid valve lower than normal causing R ventricle to be smaller causing poor flow to pulmonary vessels - usually presents after closing of ductus arteriosus

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117
Q

what 4 conditions are associated with pulmonary valve stenosis?

A

tetralogy of fallot
william syndrome
noonan syndrome and turners syndrome
congenital rubella syndrome

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118
Q

what is rheumatic fever?

A

autoimmune condition triggered by antibodies to group A beta-haemolytic strep - typically strep pyogenes causing tonsilitis. The process usually occurs 2-4 weeks after initial infection

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119
Q

what is the presentation of rheumatic fever? (7)

A

fever
joint pain - migratory of large joints
erythema marginatum rash
shortness of breath
chorea
firm painless nodules
carditis - tachy/brady, murmurs, pericardial rub, heart failure

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120
Q

what are 3 investigations for rheumatic fever?

A

throat swab and culture
Anti streptococcal antibodies (ASO) titres
ECHO, ECG, CXR

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121
Q

what criteria is used to diagnose rheumatic fever?

A

jones criteria

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122
Q

what is the jones criteria?

A

for diagnosing rheumatic fever

TWO of JONES
Joint arthritis
Organ inflammation (carditis)
Nodules
Erythema marginatum rash
Sydenham chorea

OR
One JONES and TWO FEAR
Fever
ECG changes (prolonged PR)
Arthralgia
Raised inflammatory markers

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123
Q

what is the management of rheumatic fever?

A

Abx - Benzathine benzylpenicilin or phenoxymethylpenicillin
NSAIDs or salicates (joint pain)
Aspirin and steroids - carditis
Heartfailure - Diuretics +/- ACEi
Chorea - carbamazepine

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124
Q

what are 3 complications of rheumatic fever?

A

recurrence
valvular heart disease - mitral stenosis
chronic heart failure

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125
Q

what are 6 signs of problematic reflux in babies?

A

chronic cough
hoarse cry
distress after feeding
reluctance to feed
pneumonia
poor weight gain

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126
Q

what advice can be given for GORD in babies?

A

small frequent meals
burping regularly
not overfeeding
keep baby upright after feeding

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127
Q

what kind of vomiting is present in GORD?

A

effortless mainly after feeding

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128
Q

what 3 treatments can be given in babies with GORD?

A

gaviscon mixed with feeds
thickened milk or anti-reflux formula
PPIs

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129
Q

what is a key feature of pyloric stenosis?

A

projectile vomiting

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130
Q

when does pyloric stenosis present?

A

first few weeks (4-6 weeks) of life baby failing to thrive with projectile vomiting

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131
Q

what 3 things can be found on examination of a baby with pyloric stenosis?

A

observation of stomach peristalsis
firm round ‘olive like’ mass in upper abdomen

hypochloric (low Cl-) metabolic alkalosis on blood gas

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132
Q

how is pyloric stenosis dianosed?

A

Abdo USS

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133
Q

what is the management of pyloric stenosis?

A

laparoscopic pyloromyotomy - Ramstedt’s operation

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134
Q

what are 2 common viral causes of gastroenteritits?

A

rotavirus
norovirus

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135
Q

which e.coli produces shiga toxin?

A

E.coli 0157

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136
Q

what does shiga toxin cause?

A

abdo cramps, bloody diarrhoea, vomiting - can lead to haemolytic uraemic syndrome

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137
Q

what is the most common cause of bacterial gastroenteritis?

A

campylobacter jejuni

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138
Q

where does campylobacter come from?

A

raw/improperly cooked poultry
untreated water
unpasteurised milk

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139
Q

what is the treatment of campylobacter?

A

azithromycin or ciprofloxacin

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140
Q

which bacteria causes gastroenteritis after food has not been refrigerated quickly (rice)?

A

bacillus cereus - diarrhoea resolving in 24 hours

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141
Q

what is the treatment of Giardiasis?

A

metronidazole

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142
Q

what are 4 possible complications of gastroenteritis?

A

lactose intolerance
IBS
reactive arthritis
GBS

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143
Q

what is toddlers diarrhoea?

A

chronic watery diarrhoea in well <5 year olds which can often be helped by changes in diet

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144
Q

what are the 4fs of toddler’s diets?

A

fat - shouldnt have low fat diet
Fluid - not sugary drinks, not too much water
Fruit + fruit juice - gut cannot absorb fructose or sorbitol easily
Fibre - 12-18g per day

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145
Q

what is persistent diarrhoea?

A

> 2 weeks

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146
Q

what is chronic diarrhoea?

A

> 4 weeks

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147
Q

what is encopresis?

A

foecal incontinence

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148
Q

what are 5 causes of faecal incontinence?

A

spina bifida
hirschprungs disease
cerebral palsy
overflow incontenence - stress, abuse
Constipation with overflow

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149
Q

what are 5 lifestyle factors that may contribute to constipation in children?

A

habitual not opening bowels
low fibre diet
poor fluid intake
sedentary lifestyle
psychosocial problems

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150
Q

what are 8 secondary causes of constipation in children?

A

hirschprungs disease
CF
Hypothyroid
Spinal cord lesions
sexual abuse
Intestinal obstruction
anal stenosis
cow milk intolerance

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151
Q

what is the first line laxative in children?

A

movicol

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152
Q

when is the peak incidence of apendicitis?

A

10-20 years

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153
Q

where is McBurney’s point?

A

one third of the way from the anterior superior iliac spine and the umbilicus

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154
Q

what is rovsing’s sign?

A

for apendicitis

when palpation in LIF causes pain in RIF

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155
Q

what are 6 classical features of apendicitis?

A

loss of appetite
nausea and vomiting
rovsing’s sign
guarding
rebound tenderness
percussion tenderness

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156
Q

what are 5 key differentials for apendicitis?

A

ectopic pregnancy
ovarian cysts
meckel’s diverticulum
mesenteric adenitis
appendix mass

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157
Q

what is meckel’s divertculum?

A

malformation of distal ileum present in 2% of population that can bleed, become inflamed, rupture or cause volvutus, intussusception or obstruction

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158
Q

what are 5 complications of apendicectomy?

A

bleeding, infection, pain, scars
damage to bowel or bladder
removal of normal appendix
anaesthetic risks
VTE

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159
Q

what are the 5 key features of Crohn’s? (mneumonic)

A

NESTS

No blood or mucus (less than UC)
Entire GI tract
Skip lesions
Terminal ileum most affected + Trans mural
Smoking risk factor

weight loss, strictures, fistulae

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160
Q

what are the 7 key features of UC? (mneumonic)

A

CLOSEUP

Continuous inflammation
Limited to colon and rectum
Only superficial mucosa
Smoking protective
Excrete blood and mucus
Use aminosalicylates
Primary sclerosing cholangitis

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161
Q

what are extra-intestinal manifetations of IBD?

A

finger clubbing
erythema nodosum
pyoderma gangrenosum
episcleritis and iritis
inflammatory arthritis
primary sclerosing cholangitis (UC)

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162
Q

what is one stool test that can be used for IBD but only in adults?

A

faecal calprotectin

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163
Q

what is the acute management of crohn’s?

A

1 - steroids (oral pred or IV hydrocortisone)

+ immunosuppression under specialist - azathioprine, mercaptopurine, methotrexate, infliximab

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164
Q

what is the long term management of crohn’s?

A

1 - azithioprine or mercaptopurine

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165
Q

what is the management of mild-moderate acute UC?

A

1 - aminosalcylates - mesalazine

2 - corticosteroids

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166
Q

what is the management of severe acute UC?

A

1 - IV corticosteroids (hydrocortisone)

2 - IV ciclosporin

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167
Q

what is the long term management of UC?

A

aminosalicylate - mesalazine

or azathioprine or meraptopurine

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168
Q

what is the name of surgery to remove the colon and rectum sometimes used in UC?

A

panproctocolectomy

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169
Q

what is the most common congenital abnormality of the small bowel?

A

meckel’s diverticulum

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170
Q

when do people usually present with meckel’s diverticulum?

A

<2 years

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171
Q

what are 2 antibodies in coeliac disease?

A

anti-tissue transglutaminase (anti-TTG)

anti-endomysial (anti-EMA)

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172
Q

What skin manifestation can be a sign of coeliac disease?

A

dermatitis herpetiformis - itchy blistering skin rash usually on abdomen

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173
Q

what are 3 neurological signs associated with celiac disease?

A

peripheral neuropathy
cerebellar ataxia
epilepsy

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174
Q

what are 2 findings on endoscopy in coeliac disease?

A

crypt hypertrophy
villous atrophy

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175
Q

what are 6 conditions associated with coeliac?

A

T1DM
thyroid disease
autoimmune hepatitis
primary billiary cirrhosis
primary sclerosing cholangitis
downs syndrome

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176
Q

what are 7 complications of untreated coeliac disease?

A

vitamin deficiency
anaemia
osteoporosis
ulcerative jenunitits
enteropathy associated t-cell lymphoma
non-hodgekin lymphoma
small bowl adenocarcinoma

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177
Q

what is faltering growth?

A

fall in weight across:
1+ centiles if birth weight below 9th
2+ centiles if birth weight between 9th-91st
3+ centiles if birth weight 91+ centiles

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178
Q

what are 5 causes of failure to thrive?

A

inadequate nutritional intake
difficulty feeding
malabsorption
increased energy requirements
inability to process nutrition

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179
Q

what are 5 causes of inadequate nutritional intake in children failing to thrive?

A

maternal malabsorption if breastfeeding
IDA
family or parental problems
neglect
availability of food

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180
Q

what are 4 causes of difficulty feeding?

A

poor suck (cerebral palsy)
cleft lip/palate
genetic conditions w/ abnormal face structure
pyloric stenosis

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181
Q

what are 5 causes of malabsorption in children failing to thrive?

A

CF
coeliac disease
cows milk intolerance
chronic diarrhoea
IBD

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182
Q

what are 4 causes of increased energy requirements in children failing to thrive?

A

Hyperthroidism
chronic disease - CF, CHD
malignancy
chronic infections - HIV, immunodeficiency

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183
Q

what are 2 causes of inability to process nutrients in children failing to thrive?

A

inborn errors of metabolism
T2DM

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184
Q

how do you calculate mid-parental height?

A

average of parents height

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185
Q

what is anthropometry?

A

in nutritional assessment - includes weight, height mid-upper arm circumference and skinfold thickness

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186
Q

what is marasmus?

A

severe malnutrition due to inadequate energy intake in all forms. More common <1 year olds and causes muscle wasting and loss of subcutaneous fat but no oedema

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187
Q

what is kwashiorkor?

A

Severe malnutrition due to protein deficiency but with adequate energy intake. Leads to oedema, ascites and enlarged liver with fatty infiltrates

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188
Q

what is Hirschprungs disease?

A

congenital condition where nerve cells of the myenteric (auerbach’s) plexus are absent causing a lack of peristalsis of the large bowel

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189
Q

what is the key pathopysiology of hirschprungs disease?

A

absence of parasympathetic ganglion cells in the colon

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190
Q

what are 4 syndrome that can be associated with hirschprung’s?

A

downs
neurofibromatosis
waardenburg syndrome
multiple endocrine neoplasia type II

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191
Q

what are 5 presentations of hirschprungs disease?

A

delay in passing meconium
chronic constipation since birth
abdo pain and distention
vomiting
poor weight gain and failure to thrive

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192
Q

what is hirschsprung associated enterocolitis?

A

inflammation and obstruction of the intestines in 20% of neonates with hirschprungs presenting usually between 2-4 weeks with fever, abdo distension, diarrhoea (w/ blood usually) and features of sepsis

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193
Q

what is the management of hirschsprungs associated enterocolitis?

A

Abx
fluid resuscitation
decompression of obstruction

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194
Q

how is hirschsprungs disease diagnosed?

A

rectal biopsy + histology for absence of ganglionic cells

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195
Q

what is the management of hirschsprungs disease?

A

surgical resection of aganglionic bowel

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196
Q

what age group does intussusception occur most commonly in?

A

6 months - 2 years

more common in boys

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197
Q

what is intussusception?

A

when the bowel invaginates into itself causing bowel obstruction

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198
Q

what 5 things are risk factors for intussusception?

A

concurrant viral illness
henoch-schonlein purpura
CF
intestinal polyps
meckel diverticulum

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199
Q

what are 6 presentations of intussusception?

A

sever colicky abdo pain
pale, lethargic unwell child
redcurrant jelly stool
sausage shaped RUQ mass
vomiting
intestinal obstruction

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200
Q

what is the first line investigation for intussusception?

A

USS

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201
Q

what is the treatment for intussusception?

A

1 - therapeutic enema - contrast, water or air

2- surgery

202
Q

what are 4 complications of intussusception?

A

obstruction
gangrenous bowel
perforation
death

203
Q

what are 8 causes of bowel obstruction in children?

A

meconium ileus
hirschprungs
oesophageal atresia
duodenal atresia
intussusception
imperforate anus
malrotation of the intestines + volvulus
strangulated hernia

204
Q

what can be seen on abdo xray in obstruction?

A

proximal dilated bowel loops
distal collapsed bowel loops
absence of air in rectum

205
Q

when does biliary atresia present?

A

shortly after birth

206
Q

what kind of bilirubin is high in biliary atresia?

A

conjugated bilirubin

207
Q

what is the 1st line investigation for billiary atresia?

A

conjugated and unconjugated bilirubin

208
Q

what is classed as persistent jaundice in babies?

A

> 14 days term babies
21 days premies

209
Q

what is the management of billiary atresia?

A

surgery - kasai portoenterostomy

often require full liver transplant

210
Q

what is henoch-schonlein purpura?

A

autoimmune condition usually triggered by URTI which causes inflammation of capilaries leading to purpuric rash typically on lower legs, joint pain and GI symptoms. Can also cause kidney damage

211
Q

what are the 3 key features of Henoch-schonlein purpura?

A

purpuric rash on legs
joint pain
GI symptoms

212
Q

what is an abdominal migraine?

A

severe central abdo pain >1 hour with normal examination
may also have nausea, vom, anorexia, pallor, headache, photophobia, aura

213
Q

what is the acute management of abdo migraine?

A

paracetamol
ibuprofen
sumatriptan

214
Q

what is the prophylaxis of abdo migraine?

A

1 - pizotifen - serotonin agonist

propanolol
cyproheptadine - antihistamine
flunarazine - Ca channel blocker

215
Q

what is gastroschisis?

A

a birth defect causing abdominal organs to be located outside abdomen due to defect in abdominal wall

216
Q

what are 2 examination findings in diaphragmatic hernia?

A

apex beat displaced to r side of chest
poor air entry in L chest

217
Q

what is the management of diaphragmatic hernia?

A

NG tube suction to prevent intrathoracic bowel distention and surgical repair - high mortality due to underdeveloped lungs in utero

218
Q

who are umbilical hernias more common in?

A

children of african descent

219
Q

what are 2 classes of symptoms of cow milk protein allergy?

A

GI symptoms - bloating, wind, abdo pain, diarrhoea , vomiting

Allergic symptoms - hives, angio-oedema, cough/wheeze, sneezing, watery eyes, eczema, anaphylaxis

220
Q

what formula can be used in cow milk protein allergy?

A

hydrolysed formulas or in severe cases elemental amino acid formulas

221
Q

what is the difference between cow milk intolerance and allergy?

A

intolerance has GI symptoms but no allergic symptoms unlike allergy

222
Q

what immunoglobulin causes rapid cows milk protein allergy?

A

IgE

223
Q

what are choledochal cysts?

A

congenital cyst dilations of the billary tree more commonly affecting females which have a small risk of malignancy

224
Q

what is the classic triad of choledochal cysts?

A

abdo pain
jaundice
abdo mass

225
Q

what is neonatal hepatitis syndrome?

A

idiopathic prolonged neonatal jaundice and hepatic inflammation which may cause low birth weight and faltering growth

226
Q

what is the usual presentation of colic?

A

sudden inconsolable crying/screaming accompanied by drawing knees to chest and passign excessive gas

227
Q

what 2 findings indicate pyelonephritis over uti?

A

temp >38 degrees
loin pain/tenderness

228
Q

what is the management for < 3 months with fever?

A

immediate IV Abx - cefotaxime+ amoxicillin

+ full septic screen

229
Q

what a full septic screen in <3 months?

A

bloods - FBC, CRP, cultures, lactate
urine dip
CXR
LP
? cap gas

230
Q

when should children with UTIs get an USS within 6 weeks?

A

if first UTI <6 months
recurrent UTIs
atypical utis

231
Q

what investigation can be done for defects in renal tissue and scars after UTI?

A

DMSA scan (dimercaptosuccinic acid) - injection of radioactive material using gamma camera done 4-6 months after infection

232
Q

what investigation can be done to visualise urinary anatomy, vesicouteric reflux and urethral obstruction in children <6 months?

A

micturating cystourethrogram (MCUG)
- catheterise child and inject contrast then take x rays

233
Q

what is vesico-uteric reflux?

A

where there is a developmental abnormality of the vesicouteric junction where the ureters are displaced laterally and enter the bladder directly allowing urine to reflux back up ureters and to the kidney causing dilatation and increased risk of infection and scaring

234
Q

what is the management of pyelonephritis in children >3 months?

A

oral trimethoprim 7 days or IV co-amox 2-4 days

235
Q

what is the management of UTI in children >3 months?

A

oral ABx - trimeth or nitro 3 days

236
Q

what is enuresis?

A

involuntary urination

237
Q

by what age do children usually get control of day time bladder function?

A

2 years

238
Q

by what age do children usually stop bed wetting?

A

3-4 years

239
Q

what are 5 possible causes of primary nocurnal enuresis?

A

overactive bladder - frequent small volume urination prevents development of bladder capacity
fluid intake prior to bed
failure to wake
psychological distress
secondary causes - chronic constipation, UTI, learning disability, cerebral palsy

240
Q

what is primary nocturnal enuresis?

A

wetting the bed having never stopped

241
Q

what is secodnary nocturnal enuresis?

A

wetting the bed having previously stopped for >6 months

242
Q

what are 5 causes of secondary nocturnal enuresis?

A

UTI
constipation
T1DM
psychosocial problems
abuse

243
Q

what is diurnal enuresis?

A

day time wetting self

244
Q

what are 3 management options for nocturnal enuresis?

A

Eneurisis alarm - 1st line
desmopressin (taken at bedtime)
oxybutinin - anticholinergic
imipramine - tricyclic antidepressant

245
Q

what age range is nephrotic syndrome most common in?

A

2-5 years

246
Q

what is the classical triad of nephrotic syndrome?

A

low serum albumin
high urine protein (3+ or >3 grams)
oedema

247
Q

what are 6 signs of nephrotic syndrome?

A

low serum albumin
high urine protein
oedema
deranged lipid profile
high BP
hyper-coagulability

248
Q

what is the most common cause of nephrotic syndrome in children?

A

minimal change disease

249
Q

what are 4 secondary causes of nephrotic syndromes?

A

intrinsic kidney disease - focal segmental glomerulosclerosis, mebranoproliferative glomerulonephitis

Henoch schonlein purpura
diabetes
infection - HIV, hepatitis, malaria

250
Q

what can be seen on urinalysis in minimal change?

A

small molecular weight proteins and hyaline casts

251
Q

what is the first line management of minimal change disease?

A

high dose corticosteroids - prednisolone

252
Q

what is the management of steroid resistant nephrotic syndrome?

A

ACEi
immunosupressants - cyclosporine, tacrolimus, rituximab

253
Q

what is the general management of nephrotic syndromes (5)?

A

high dose steroids - pred
low salt diet
diuretics
albumin infusion
antibiotic prophylaxis

254
Q

what are 5 complications of nephrotic syndromes?

A

hypovolaemia and low BP
thrombosis
infection - kidneys leak immunoglobulins
acute or chronic renal failure
relapse

255
Q

what is the pathophysiology of nephrotic syndromes?

A

the basement membrane of the glomerulus becomes highly permeable to proteins allowing them to leak from the blood into the urine

256
Q

what is the pathophysiology of nephritic syndromes?

A

inflammation of the nephrons

257
Q

what are 3 consequences of nephritic syndromes?

A

reduction in kidney function
haematuria
proteinuria (less than nephrotic)

258
Q

what are the two most common causes of nephritis in children?

A

post-streptococcal glomerulonephritis
IgA nephropathy

259
Q

what is the pathophysiology of post-streptococcal glomerulonephritis?

A

1-3 weeks after b-haemolytic strep (strep pyogenes) infection e.g tonsilitis immune complexes get stuck in the glomeruli and cause inflammation leading to AKI

260
Q

what 2 investigations can be done to test for strep in post-strep glomerulonephritis?

A

positive throat swab
anti-streptolysin antibody titres

261
Q

what is the management of nephritis?

A

supportive mainly

manage complications - IgA may need immunosupression

262
Q

what is IgA nephropathy a complication of?

A

Henoch-schonlein purpura (IgA vasculitis)

263
Q

what is the pathophysiology of IgA nephropathy?

A

IgA deposits in nephrons cause inflammation

264
Q

what 2 things can be seen on biopsy in IgA nephropathy?

A

IgA deposits
Glomerular mesangial proliferation

265
Q

what age group is usually affected with IgA nephropathy?

A

teens and young adults

266
Q

what usually triggers haemolytic uraemic syndrome?

A

shinga toxin (from e.coli 0157 or shingella)

267
Q

what is the classical triad of haemolytic uraemic syndrome?

A

haemolytic anaemia
AKI
thrombocytopenia

268
Q

what increases the risk of haemolytic uraemic syndrome?

A

use of antibiotics and loperamide to treat gastroenteritis caused by e.coli0157 or shingella

269
Q

how long after onset of gastroenteritis do symptoms of haemolytic uraemic syndrome usually start?

A

5 days

270
Q

what are 8 presentations of haemolytic uraemic syndrome?

A

reduced urine output
haematuria or dark brown urine
abdo pain
lethargy and irritablility
confusion
oedema
HTN
bruising

271
Q

what is the management of haemolytic uraemic syndrome?

A

supportive - renal dialysis, antihypertensives, maintain fluid balance, blood transfusion

272
Q

what is hypospadias?

A

condition where uretheral meatus is displaced to underside of penis

273
Q

what is epispadias?

A

condition where the urethral meatus is displaced to the top side of the penis

274
Q

what is chordee?

A

where head of penis is bent downwards

275
Q

what are 3 complications of hypospadias?

A

difficulty directing urination
cosmetic and psychological concerns
sexual dysfunction

276
Q

what is phemosis?

A

pathological non-retration of foreskin

277
Q

what condition is the most common cause of phimosis?

A

balantitis xerotica obliterans

278
Q

what reduction in renal function classes as AKI?

A

<0.5 ml/Kg/hour over 6 hours

279
Q

what category of cause is most common in childhood AKI?

A

pre-renal

280
Q

what are the 2 most common causes of intra-renal failure in children?

A

haemolytic uraemic syndrome
acute tubular necrosis

281
Q

what is stage 1 ckd?

A

eGFR >90 ml/min per 1.73 m2

282
Q

what is stage 2 ckd?

A

eGFR 60-89

283
Q

what is stage 3 ckd?

A

eGFR 30-59

284
Q

what is stage 4 eGFR?

A

eGFR 15-29

285
Q

what is stage 5 ckd?

A

eGGR <15 ml/min per 1.73m2

286
Q

what 8 presenting features of severe CKD?

A

anorexia and lethargy
polydipsia and polyuria
faltering growth
bony deformities from renal rickets
hypertension
acute on chronic renal failure
proteinuria
normochomic normocytic anaemia

287
Q

what type of hypersensitivity reaction in anaphylaxis?

A

type 1

288
Q

what immunoglobulin causes anaphylaxis?

A

IgE

289
Q

what is the pathophysiology of anaphylaxis?

A

igE stimulates mast cells to rapidly release histamine in mast cell degranulation

290
Q

what is the key feature of anaphylaxis vs non-anaphylactic allergy?

A

anaphylaxis causes compromise of airway, breathing or circulation

291
Q

what are the 4 allergic symptoms?

A

urticaria
itching
angio-oedema
abdominal pain

292
Q

what is the management of anaphylaxis?

A

ABCDE

IM adrenaline
Antihistamines - chlorphenamine or certirizine
steroids - hydrocortisone

293
Q

what can be a complication of anaphylaxis?

A

biphasic reaction - second anaphylactic reaction after treatment

294
Q

what investigation can be done for anaphylaxis?

A

serum mast cell tryptase - within 6 hours of event

295
Q

what are 5 reasons someone with a non-anaphylactic allergic reaction may get an epipen?

A

asthma requiring ICS
poor access to medical tx
adolescents - higher risk
nut/sting allergies
significant co-morbidities

296
Q

what is the skin sensitisation theory of allergy?

A

there is a break in the infants skin that allows allergens from the environment to react with the immune system but there is no contact with the allergen through the GI tract

297
Q

what is the classification system for hypersensitivity reactions?

A

cooms and gell

298
Q

what is a type 1 hypersensitivity reaction?

A

IgE antibodies trigger mast cells and basophils to degranulate causing an immediate reaction - food allergy reactions

299
Q

what is a type 2 hypersensitivity reaction?

A

IgG and IgM mediated activating the complement system leading to direct damage of local cells - haemolytic disease of newborn, transfusion reactions

300
Q

what is a type 3 hypersensitivity reaction?

A

immune complexes accumulate and cause damage to local tissues - autoimmune conditions

301
Q

what is a type 4 hypersensitivity reaction?

A

cell mediated caused by T lymphocytes which are inappropriately activated causing inflammation and damage to local tissues - organ transplant rejection, contact dermatitis

302
Q

what are 3 investigations for allergies?

A

skin prick test
RAST testing - measure of allergen specific IgE
food challenge - gold standard

303
Q

what hypersensitivity reaction is allergic rhinits?

A

type 1 IgE

304
Q

what is the presentation of allergic rhinitis?

A

runny, bocked, itchy nose
sneezing
itchy, red, swollen eyes

305
Q

what are 3 non-sedating antihistamines?

A

certirizine
loratadine
fexofenadine

306
Q

what are 2 sedating anti-histamines?

A

chlorpenamine
promethazine

307
Q

what can be taken as prophylactic for allergic rhinits?

A

nasal corticosteroids - fluticasone, mometasone

308
Q

what are 3 live vaccinations?

A

BCG
MMR
nasal flu vaccine

309
Q

where is eczema usually found?

A

flexor surfaces

310
Q

what are 2 thick emolients?

A

50;50 ointment
hydromol

311
Q

what is the steroid cream ladder?

A

mild - hydrocortisone 0.5,1,2.5%
moderate - eumovate 0.05%
potent - betamethasone 0.1%
very potent - clobetasol propionate 0.05%

312
Q

what is the most common bacterial infection in eczema?

A

s aureus

313
Q

what abx can be used to treat bacterial infection of eczema?

A

flucloxacillin

314
Q

what is eczema herpeticum?

A

viral skin infection caused by HSV-1 or varicella zoster

315
Q

what is the presentation of eczema herpeticum?

A

eczema sufferer developed widespread painful vesicular rash with fever, lethargy, irritability, reduced oral intake and swollen lymph nodes

316
Q

What is the management of eczema herpeticum?

A

aciclovir

317
Q

what is stevens-johnson syndrome and toxic epidermal necrosis?

A

a disproportionate immune response causing epidermal necrosis resulting in blistering and shedding of top layer of skin - SJS <10% of body, TEN >10% of body

318
Q

what are 5 medications that can cause stevens-johnson syndrome?

A

anti-epileptics(lamotrigine, phenotoin)
antibiotics
allopurinol
NSAIDs
Penicillin

319
Q

what are 7 infections that can cause stevens-johnson syndrome?

A

herpes
HIV
Mumps
FLu
EBV
mycoplasma pneumonia
CMV

320
Q

what is the natural history of stevens-johnson syndrome?

A

starts with fever, cough, sore throat, mouth, eyes and skin
then develop purple/red rash which blisters
the after a few days skin sheds leaving raw tissue underneath

can also affect internal organs

321
Q

what is the management of stevens-johnson syndrome?

A

admit to derm/burns unit

steroids
immunoglobulins
immunosuppression

322
Q

what are 3 complications of stevens-johnson syndrome?

A

secondary infection
permanent skin damage and scarring
visual complications with eye involvement

323
Q

what are 6 triggers for acute urticaria?

A

allergies
contact with chemicals, latex or nettles
medications
viral infection
insect bites
rubbing skin

324
Q

what are the 3 different types of chronic urticaria?

A

chronic idiopathic urticaria
chronic inducible urticaria
autoimmune urticaria

325
Q

what are 6 triggers for chronic inducible urticaria?

A

sunlight
temperature change
exercise
strong emotions
hot/cold weather
pressure

326
Q

what is the management of urticaria?

A

Antihistamines - fexofenadine

if problematic - anti-leukotrienes (montelukast), omalizumab, cyclosporin

327
Q

what can cafe-au-lait spots be a sign of?

A

neurofibromatosis type 1 (if have 6+)

328
Q

what 3 things can port wine marks rarely be a sign of?

A

sturge-weber syndrome
klippel trenaunay syndrome
macrocephaly-capillary malformation

329
Q

what are mongolial blue spots?

A

blue/black macular discolouration at base of spine and on buttocks or thighs - fade over first few years of life

330
Q

what is osteogenesis imperfecta?

A

genetic condition also known as brittle bone disease caused by mutation in formation of collagen

331
Q

what are 8 features associated with osteogenesis imperfecta?

A

hypermobility
blue/grey sclera
triangular face
short stature
deafness from early adulthood
dental problems
bone deformities
joint and bone pain

332
Q

what is the pathophysiology of rickets?

A

deficiency in vitamin D/calcium which results in defective bone mineralisation

333
Q

what is the name of the genetic version of rickers?

A

hereditary hypophasphataemic rickets

334
Q

what are 8 presentations of rickets?

A

lethargy
bone pain
swollen wrists
bone deformuty
poor growth
dental problems
muscle weakness
pathological or abnormal fractures

335
Q

what are 5 bone deformities in rickets?

A

bowing of legs
knock knees
rachitic rosary - expanded ribs causing lumps on chest
craniotabes - soft skull, delayed closing
delayed teeth

336
Q

what investigation is done for vitamin d deficiency?

A

serum 25-hydroxyvitamin D - <25nmol/L = deficiency

337
Q

what are 5 investigations that can be done for rickets?

A

serum 25-hydroxyvitamin D
serum calcium
serum phosphate
serum alkaline phosphatase
parathyroid hormone

338
Q

what is the most common cause of hip pain in children 3-10 years?

A

transient synovitis

339
Q

what is transient synovitis often associated with?

A

preceeding viral upper resp tract infection

340
Q

what age is septic arthitis most common in?

A

<4 years

341
Q

what is teh most common causative organism of septic arthritis?

A

staph aureus

342
Q

what are 4 investigations of the synovial fluid in septic arthritis?

A

gram staining
crystal microscopy
culture
antibiotic sensitivities

343
Q

what is the most common causative organism of osteomyelitis?

A

s.aureus

344
Q

what are 6 risk factors for osteomyelitis?

A

open fracture
orthopaedic surgery
immunocompromised
sickle cell
HIV
TB

345
Q

what is perthes disease?

A

disruption of blood flow to femoral head causing avascular necrosis of the epiphysis of the femur

346
Q

what age group does perthes disease occur most commonly in?

A

5-8 years - mostly in boys

347
Q

what is slipped upper femoral eiphysis? (SUFE)

A

when head of femur displaces along growth plate

348
Q

who is SUFE more common in?

A

boys aged 8-15
obese children

349
Q

how do people with SUFE prefer to hold their hip?

A

in external rotation and have limited movement of hip

350
Q

what is the management of SUFE?

A

surgery to correct femoral head position

351
Q

what is osgood-schlatter disease?

A

inflammation of the tibial tuberosity where patella ligament inserts causing anterior knee pain in adolescents - usually unilateral but can be bilateral. causes hard lump at front of knee

352
Q

what are 3 presentations of osgood-schlatter?

A

visible or palpabl hard tender lump at tibial tuberosity
pain in anterior knee
pain exacerbated by activity, kneeling or extension of knee

353
Q

what is a complication of osgood-schlatters?

A

avulsion fracture - tibial tuberosity separated from tibia

354
Q

what is the management of osgood-schlaters?

A

reduction in physical activity
ice
NSAIDs

355
Q

what is developmental dysplasia of the hip?

A

structural abnormality n developmental of foetal bones leading to instability of te hips and tendency for sublaxation or dislocation

356
Q

what are 7 risk factors for developmental dysplasia of the hip?

A

FHx 1st degree relative
breech presentation 36 weeks
multiple pregnancy
female
oligohydramnios
prematurity
macrosomnia

357
Q

what are 5 findings that may suggest developmental dysplasia of the hip on neonatal examination?

A

different leg lengths
restricted hip abduction on one side
significant bilateral restriction in hip abduction
difference in knee level with flexed hips
clunking of hips

358
Q

what are two special test for developmental dysplasia of the hio?

A

ortolani test
barlow test

359
Q

what is the ortolani test?

A

for developmental dysplasia of the hip

hips and knees flexed, hips gently abducted and pushed from the back to see if they will dislocate anteriorly

360
Q

what is barlow test?

A

for developmental dysplasia of the hip

hips and knees flexed and gentle pressure put on knees through femur to see if hips will dislocate posteriorly

361
Q

what investigation can be done for developmetal dysplasia of the hips?

A

USS

362
Q

what is the management for developmental dysplasia of the hips?

A

Pavlik harness (<6 months) for 6-8 weeks

surgery if harness fails or >6 months

363
Q

what are the 5 subtypes of juvenile idiopathic arthritis?

A

systemic JIA
polyarticular JIA
Oligoarticular JIa
enthesitis related arthritis
juvenile psoriatic arthritis

364
Q

what is systemic juvenile idiopathic arthritis also known as?

A

Still’s disease

365
Q

what are 8 manifestations of systemic JIA?

A

subtle salmon pink rash
high swinging fevers
enlarged lymph nodes
weight loss
joint inflammation and pain
splenomegally
muscle pain
pleuritis and pericarditis

366
Q

what is are 2 key complication of systemic JIA?

A

macrophage activation syndrome - severe inflammatory responce causing DIC, anaemia, thrombocytopenia, bleeding, non-blanching rash

chronic anterior uveitis

367
Q

what are 4 key non-infective causes of fever in children > 5 days?

A

kawasaki disease
still disease (systemic JIA)
rheumatic fever
leukaemia

368
Q

what is polyarticular JIA?

A

idiopathic inflammatory arthritis in 5+ joints - equivalent of RhA in adults though most children are seronegative

369
Q

what is oligoarticular JIA?

A

monoarthritis affecting <4 joints, classically associated with anterior uveitis. most common in gils under 6

370
Q

what is enthesitis related arthritis?

A

paediatric seronegative spondyloarthropathies. inflammatory arthritis + enthesitis (inflammation of tendon insertion points)

usually HLA-B27 +ve

371
Q

what are 5 signs of juvenile psoriatic arthritis?

A

places of psoriasis
nail piitting
onycholysis (seperation of nail bed)
dactylitis
enthesitis

372
Q

what is the management of juvenile idiopathic arthritis?

A

NSAIDs
steroids
DMARDs
Biologics - TNF inhibitors, infliximab etc

373
Q

what age group does kawasaki’s usually affect?

A

<5 years

374
Q

what is a key complication of kawasaki disease?

A

coronary artery aneurysm

375
Q

what is the pathophysiology of kawasaki disease?

A

systemic medium sized vessel vasculitis?

376
Q

what are 7 key features of kawasaki?

A

high (>39) fever >5 days
widespread erythematous maculopapular rash on trunk
Oedema of hands and feed preceeding desquamation
Strawberry tongue and cracked lips
cervical lymphadenopathy
bilateral conjunctivitis
arthritis

377
Q

what are 5 investigations for kawasaki?

A

FBC - anaemia, leukocytosis, thrombocytosis
LFTS - hypoalbuminaemia, elevated enzymes
raised inflammatory markers (particularly esr)
Urinaralysis - wt cells without infection
ECHO

378
Q

what happens in the acute phase of kawasakis?

A

most unwell for 1-2 weeks
Fever, rash, lymphadenopathy

379
Q

what happens in the subacute phase of kawasakis?

A

weeks 2-4
acute symptoms settle
desquamation, strawberry tongue and arthralgia
occur - there is risk of coronary artery aneurysms forming

380
Q

what happens in the covalescent stage of kawasakis?

A

week 2-4
remaining symptoms settle

381
Q

what is the management of kawasaki disease?

A

high dose aspirin - reduce risk of throbosis
IV Ig to reduce risk of coronary artery aneurysm

382
Q

what is Reye’s syndrome

A

a preceeding viral infection usually treated with ASPIRIN followed by acute encephalopathy and hepatic dysfunction in children and young people

383
Q

what follow up is needed with kawasakis?

A

ECG and ECHO

384
Q

what cells produce surfactant?

A

type II pneumocytes

385
Q

when does surfactant start to be produced?

A

between 24-34 weeks gestation

386
Q

what is required to keep the ductus arteriosus open?

A

prostaglandins

387
Q

what can extended hypoxia lead to during birth?

A

hypoxic-ischaemic encephalopathy => cerebral palsy

388
Q

what are 3 issues of neonatal resuscitation?

A

large SA to weight ratio - cold
born wet and loose heat rapidly
meconium aspiration

389
Q

what are 5 principles of neonatal resuscitation?

A

warm baby
calculate APGAR score
stimulate breathing
inflation breaths
chest compressions

390
Q

what can be used to manage hypoxic ischaemic encephalopathy?

A

therapeutic hypothermia

391
Q

when should you calculate the APGAR score?

A

1, 5 and 10 minutes during resus

392
Q

how do you stimulate breathing in neonatal resussitation?

A

dry vigorously with towel
place baby head in neutral
check for airway obstruction and consider aspiration if gasping or unable to breathe

393
Q

how should inflation breaths be given in neonates?

A

2 cycles of 5 inflation breaths
if no response 30s of ventilation breaths can be used
then chest compressions coordinated with ventilation breaths

394
Q

what should be used for inflation breaths in preterm vs term babies?

A

preterm - air and O2
term - just air

395
Q

when should you start chest compressions in a neonate?

A

if HR <60 bpm despite resus and inflation breaths

396
Q

what is the APGAR score?

A

for neonatal resus

Appearance (skin colour)
Pulse
Grimmace (to stimulation)
Activity (tone)
respiration

397
Q

what is the scoring for appearance in APGAR?

A

blue/pale centrally = 0
blue extremities = 1
pink = 2

398
Q

what is the scoring for pulse in APGAR?

A

Absent = 0
<100 = 1
>100 = 2

399
Q

what is the scoring for grimmace in APGAR?

A

no response = 0
little response = 1
good response = 2

400
Q

what is the scoring for activity in APGAR?

A

floppy = 0
flexed arms and legs =1
active = 2

401
Q

what is the scoring for respiration in APGAR?

A

absent =0
slow/irregular = 1
strong/crying = 2

402
Q

how long should delayed cord clamping be?

A

1 minute

403
Q

under what gestation can be affected by respiratory distress syndrome?

A

< 32 weeks

404
Q

what xray changes are seen in respiratory distress syndrome?

A

ground glass appearance

405
Q

what are 4 types of ventilatory support that may be needed by premature neonates?

A

intubation and ventilation
endotracheal surfactant
CPAP
supplementary oxygen

406
Q

what are 6 short term complications of respiratory distress syndrome?

A

penumothorax
infection
apnoea
intraventricular haemorrhage
pulmonary haemorrhage
necrotising enterocolitis

407
Q

what are 3 long term complications of respiratory distress syndrome?

A

chronic lung disease of prematurity
retinopathy of prematurity
neurological, hearing or visual impairment

408
Q

what are 4 causes of hypoxic ischaemic encephalopathy?

A

maternal sock
intrapartum haemorrhage
prolapsed cord
nucal cord

409
Q

what is the staging for hypoxic ischaemic encephalopathy called?

A

sarnat staging

410
Q

what is the presentation of mild hypoxic ischaemic encephalopathy?

A

poor feeding, generally irritable, hyper-alert

resolves within 24 hours
normal prognosis

411
Q

what is moderate hypoxic ischaemic encephalopathy?

A

poor feeding, lethargy, hypotonic, seizures

can take weeks to resolve
40% develop cerebral palsy

412
Q

what is severe hypoxic ischaemic encephalopathy?

A

reduced consciousness, apnoea, flaccid, reduced or absent reflexes
50% mortality
90% develop cerebral palsy

413
Q

what temperature is therapeutic hypothermia cooled to and for how long?

A

33-34 degrees for 72 hours

414
Q

when can physiological jaundice be present?

A

from 2-10 days of age

415
Q

what are 8 causes of increased production of bilirubin in neonates?

A

haemolytic disease of newborn
ABO incompatibility
Haemorrhage
intraventricular haemorrhage
cephalo-haemorrhage
polycythaemia
sepsis and DIC
G6PD deficiency

416
Q

what are 6 causes of decreased bilirubin clearance in neonates?

A

prematurity
breast milk jaundice
neonatal cholestasis
extrahepatic biliary atresia
endocrine disorders
gilbert syndrome

417
Q

when is jaundice always pathological?

A

in first 24 hours of life

can be sign of sepsis

418
Q

what is kernicterus?

A

brain damage due to high unconjugated bilirubin

419
Q

what causes haemolytic disease of the newborn?

A

rhesus incompatability

420
Q

what is prolonged neonatal jaundice?

A

14+ days in full term babies
21+ days in premature babies

421
Q

what test is done for autoimmune haemolysis?

A

direct coombs test

422
Q

what is used to monitor neonatal jaundice?

A

treatment threshold charts

423
Q

what is the usual treatment of neonatal jaundice?

A

phototherapy with blue light (450nm)

424
Q

what are 3 complications of kernictus?

A

cerebral palsy
learning disability
deafness

425
Q

what is the medical management of supraventricular tachycardia?

A

adenosine - after valsalva manoeuvres

426
Q

what is necrotising enterocolitis?

A

disorder affecting premature neonates where part of bowel becomes necrotic and can lead to perforation

427
Q

what are 5 risk factors for necrotising enterocilitis?

A

very low birth weight or v premature
formula feeds
resp distress and assisted ventilation
sepsis
patent ductus arteriosus and congenital heart disease

428
Q

what are 6 features of necrotising enterocolitis?

A

intolerance to feeds
vomiting green bile
distended tender abdomen
blood in stool
absent bowel sounds
generally unwell

429
Q

what investigation is good for diagnosing necrotising enterocolitis?

A

abdo Xray - supine and lateral

430
Q

what can be seen on xray with necrotising enterocolitis?

A

dilated bowel loops
bowel wall oedema
pneumatosis intestinalis - gas in bowel wall
pneumoperitoneum - free gas in peritoneum
gas in portal veins

431
Q

what is the management of necrotising enterocolitis?

A

nil by mouth
iv fluids
total parenteral nutrition
antibiotics
NG tube - to drain fluid and gas

SURGERY

432
Q

what are 8 complications of necrotising enterocolitis?

A

perforation and peritonitis
sepsis
death
strictures
abcess formation
recurrence
long term stoma
short bowel syndrome

433
Q

what are the torch congenital infections?

A

Toxoplasmosis
rubella
cytomegalavirus
herpes simplex
HIV

434
Q

at what gestation is the risk of congenital rubella highest?

A

< 3 months

435
Q

what are 3 features of congenital rubella?

A

Classic triad of - deafness, blindness (congenital cataracts) and congenital heart disease

also learning disability

436
Q

what are 6 features of congenital cytomegalovirus?

A

foetal growth restriction
microcephaly
hearing loss
vision loss
learning disability
seizures

437
Q

what is the classic triad of congenital toxoplasmosis gondii?

A

intracranial calcification
hydrocephalus
chorioretinitis (type of posterior uveitis)

438
Q

what are 6 manifestations of neonatal herpes?

A

external herpes lesions
liver involvement
encephalitis
sezures, tremmors, lethargy, irritability

439
Q

what are 5 complications of chicken pox in pregnancy?

A

foetal varicellar zoster syndrome
pneumonitis
hepatitis
encephalitis
severe neonatal infection

440
Q

what are 6 mamifestations of congenital varicella syndrome?

A

foetal growth restriction
microcephaly
hydrocephalus, learnign disability
scars and skin changes
limb hypoplasia
cataracts and chorioretinitis

441
Q

what is given to a baby immediatly after birth?

A

vitamin K IM injection

442
Q

what 9 conditions are tested for in the blood spot test?

A

sickle cell
CF
congenital hypothyroidism
phenylketonuria
medium chain acy-COA dehydrogenase deficiency
maple syrup urine disease
isovaleric acidaemia
glutaric aciduria type 1
homocystin

443
Q

at what age does the heel prick test happen?

A

5 days old

444
Q

how long does the heel prick take to come back?

A

6-8 weeks

445
Q

when is the NIPE examination done?

A

<72 hours of birth

446
Q

when is the NIPE repeated?

A

6-8 weeks by GP

447
Q

when does the ductus arteriosus close?

A

1-3 days

448
Q

how small should the difference in pre-ductal and post-ductal O2 saturations be in the NIPE?

A

<2%
pre-ductal - R hand
post ductal - feet

449
Q

what is the name of oedema of the scalp outside the periosteum due to trauma in birth which crosses suture lines?

A

caput succedaneum

450
Q

what is the name of a collection of blood between the skull and periosteum due to trauma in delivery which does not cross suture lines?

A

cephalohaematoma

451
Q

what is erbs palsy?

A

damage to C5/6 brachial plexus ileading to lweakness in shoulder abduction and external rotation, arm flexion and finger extension

may have a ‘waiters tip appearance’

452
Q

what are 6 risk factors for neonatal sepsis?

A

vaginal GBS colonisation
GBS sepsis in previous baby
Maternal sepsis, chorioamniotitis or fever
prematurity
premature ROM
prolonged rupture of membranes

453
Q

what are 6 red flags for neonatal sepsis?

A

confirmed/suspected sepsis in mother
signs of sock
seizures
term baby needing mechanical ventilation
resp distress starting 4+ hours after burth
presumed sepsis in other multiple

454
Q

what are 10 clinical features of neonatal sepsis?

A

fever
reduced tone and activity
poor feeding
resp distress or apnoea
vomiting
tachy or brady
hypoxia
jaundice <24 hours
seizures
hypoglycaemia

455
Q

what is the nice treatment for neonatal sepsis?

A

monitor if 1 risk factor/clinical feature

start antibiotics within one hour if 2 risk factors/clinical features or 1 red flag

456
Q

what antibiotic should be given in neonatal sepsis?

A

benzylpenicillin or gentamycin

cefotaxime if lower risk

457
Q

what is the ongoing management of neonatal sepsis?

A

check CRP at 24 hours and blood cultures at 36 hours

consider stopping Abx if blood cultures negative and CRP <10 at day 2

458
Q

what counts as extreme preterm?

A

<28 weeks

459
Q

what counts as very preterm?

A

28-32 weeks

460
Q

what counts as moderate/late preterm?

A

32-37 weeks

461
Q

what are 10 early complications of prematurity?

A

respiratory distress syndrome
hypothermia
hypoglycaemia
poor feeding
apnoea and bradycardia
neonatal jaundice
intraventricular haemorrhage
retinopathy of prematurity
necrotising enterocolitis
immature immune system and infection

462
Q

what are 5 long term complications of prematurity?

A

chronic lung disease of prematurity
learnign an dbehvioural difficulties
susceptibility to infections - particularly resp
hearing and visual impairement
cerebral palsy

463
Q

what is apnoea?

A

stopping breathing >20 seconds or shorter periods with oxygen desaturation or bradycardia

464
Q

what is the causes of apnoea in neonates?

A

due to immaturity of the autonomic nervous system

465
Q

what is the management of neonatal apnoea?

A

tactile stimulation
IV caffeine

466
Q

what is the pathophysiology of retinopathy of prematurity?

A

retinal blood vessel formation is stimulated by hypoxia which is the normal condition of the retina during pregnancy so with early exposure to oxygen in prematurity this can stop retinal blood vessel development and cause retinal detachment

467
Q

who should screening for retinopathy of prematurity be offered to?

A

<32 weeks gestation
<1.5kg

468
Q

what is the management of retinopathy of prematurity?

A

transpupillary laser photocoagulation

cyotherapy
injections of intravitreal VEGF inhibitors
Surgery if retinal detachment

469
Q

how is a diagnosis of meconium aspiration confirmed?

A

CXR

470
Q

what are 3 risk factors for meconium aspiration?

A

gestational age >42 weeks
foetal distress
APGAR score <7

471
Q

what is the management for suspected HSV encephalitis?

A

aciclovir

472
Q

How do you get infected with listeria?

A

consumption of dairy products, raw vegetables, meats and refrigerated foods

passed from mother to baby placentally

473
Q

what are clinical manifestations of neonatal listeriosis?

A

abortion
prematurity
still birth
neonatal sepsis

474
Q

how can listeria infection be diagnosed prenatally and postnatally?

A

culture or PCR of blood, cervix or amniotic fluid of mother prenatally

culture/PCR of blood, CSF, gastric aspirate, meconium of neonate

475
Q

what is the management of neonatal listeriosis?

A

Ampicillin +/- gentamicin

476
Q

what is maternal hyperthyroidism associated with?

A

foetal tachycardia
small for gestational age
prematurity
still birth
congenital malformations

477
Q

what is maternal hypothyroidism associated with?

A

lower IQ and impaired psychomotor development

478
Q

what are 6 risk factors for neonatal hypoglycaemia?

A

IUGR
preterm
born to mothers with diabetes
hypothermic
polycythaemic
ill for any reason

479
Q

what are 6 symptoms of neonatal hypoglycaemia?

A

jitters
irritability
apnoea
lethargy
drowsiness
seizures

480
Q

what counts as hypoglycaemia in neonates?

A

<2.6 mmol/L

481
Q

what is the pathophysiology of cleft lip and palate?

A

lip - failure of fusion of the frontonasal and maxillary processes
Palate - failure of fusion of palatine processes and nasal septum

482
Q

when is a cleft lip and palate usually fixed?

A

lip - 3 months
palate - 6 months - 1 year

483
Q

what are 2 USS signs in pregnancy of oesophageal atresia?

A

polyhydramnios
no stomach bubble

484
Q

what is a clinical sign of oesophageal atresia in neonates?

A

persistant salivation and drooling

485
Q

what is the name of a group of midline congenital defects like oesophageal atresia?

A

VACTERL association

Vertebral
anorectal
cardiac
tracheo-oesophageal
renal
limb

anomalies

486
Q

what is the management of oesophageal atresia?

A

continuous suction of secretions to avoid aspiration until surgery is available

487
Q

what is an exomphalos?

A

where abdominal contents protrude through umbilical ring covered by transparent sac

488
Q

what congenital condition is duodenal atresia associated with?

A

downs syndrome

489
Q

what is an x-ray/USS sign of duodenal atresia?

A

‘double bubble’ of of distention of stomach and duodenal cap with absence of air distally

490
Q

what are 3 obstetric complications of diabetes in pregnancy?

A

polyhydramnios
preeclampsia
early and late foetal loss

491
Q

what are 3 foetal complications of diabetes in pregnancy?

A

congenital malformations
IUGR
macrosomia

492
Q

what are 4 neonatal complications of diabetes in pregnancy?

A

neonatal hypoglycaemia
respiratory distress syndrome
hypertrophic cardiomyopathy
polycythaemia

493
Q

what is scoliosis?

A

lateral curvature of frontal plane of spine. In severe cases can lead to cardiorespiratory failure from curvature of chest

494
Q

what is torticollis?

A

Head twisting to the side also called wry neck. The most common cause in infants is a sternomastoid tumour.

495
Q

what is the incubation period for mumps?

A

14-25 days

496
Q

what is the presentation of mumps?

A

prodromal fever, muscle aches, lethargy, reduced appetite, headache and dry mouth for a few days

followed by parotid swelling

usually self limiting after around a week

497
Q

what are 4 complications of mumps?

A

pancreatitis
orchitis
meningitis
sensorineural hearing loss

498
Q

How is mumps diagnosed?

A

salivary PCR
salivary or blood antibodies

499
Q

what is the management for mumps?

A

supportive - self limiting infection

500
Q

what is the rash and its spread in chicken pox?

A

widespread erythematous raised vesicular blistering lesions - starts as macules then papules then vesicles
Starts on trunk or face and spreads outwards over 2-5 days the crusts after 5 days